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Congenital Heart Disease Huang et al

The potential of disproportionate growth of tricuspid valve after


decompression of the right ventricle in patients with pulmonary
atresia and intact ventricular septa
Shu-Chien Huang, MD,a Kozo Ishino, MD,b Shingo Kasahara, MD,b Ko Yoshizumi, MD,b
Yasuhiro Kotani, MD,b and Shunji Sano, MDb

Objective: Tricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with
intact ventricular septum. Lack of right ventricle–pulmonary artery continuity is associated with poor tricuspid
valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth
of the tricuspid valve after establishment of right ventricle–pulmonary artery continuity.
Methods: We studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial
right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter
(Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before
definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with
the changes of Z-values (Zt2 ! Zt1) were analyzed.
CHD

Results: The mean initial tricuspid Z-value (Zt1) was !6.2 " 3.5. After treatment (Zt2), the mean Z-value was
!6.0 " 3.4 (n ¼ 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 !
Zt1) was larger than þ2 in 11 (32%) patients and smaller than !2 in 6 (18%) patients. Increases in Z-value
(Zt2 ! Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured
after initial palliation (r ¼ !0.54; P ¼ .001) and the initial tricuspid valve Z-value (Zt1) (r ¼ !0.40; P ¼ .02).
Conclusions: Disproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid
valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates
with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery.

Pulmonary atresia with an intact ventricular septum Previous studies of PA–IVS showed that RV and tricuspid
(PA–IVS) is an uncommon congenital heart disease with valve diameters had poorer growth when RV–pulmonary
a variable degree of right ventricular (RV) hypoplasia and artery continuity was not achieved.1-3 For better RV growth,
coronary artery anomalies. Patients with RV-dependent cor- the initial RV outflow procedure (surgically or percutane-
onary circulation, which contradicts decompression of the ously) with or without systemic–pulmonary shunting is the
RV, should be better managed with single ventricular circu- most commonly used repair strategy.4-7
lation (Fontan-type repair). Otherwise, achieving biventri- There are many reports on increased RV volume after
cular repair with good functional status and low systemic surgical or catheter-based RV decompression procedures,
venous pressure is the ideal goal. For biventricular circula- but there is a paucity of similar data describing the growth
tion to be achieved, the tricuspid valve and RV must have of tricuspid valves.2,5,8-10
adequate size to support pulmonary blood flow. Inasmuch as a small tricuspid valve causes limited RV
inflow and the tricuspid valve diameter is considered the
determinant for patients to undergo single ventricle or bi-
From the Department of Cardiovascular Surgery,a National Taiwan University Hospi- ventricular repair, we reviewed the data at our institution
tal, Taipei, Taiwan, and the Department of Cardiovascular Surgery,b Okayama to see whether the tricuspid valve had ‘‘disproportionate
University Hospital, Okayama University Graduate School of Medicine and
Dentistry, Okayama, Japan.
enlargement’’ compared with somatic growth after decom-
Shu-Chien Huang was supported by a grant from National Taiwan University Hospi- pression of the RV. We also wanted to identify the factors
tal, Taipei, Taiwan, for study in Okayama, Japan, from April 2006 to September associated with growth of the tricuspid valve after RV de-
2006.
Received for publication Oct 24, 2008; revisions received Feb 26, 2009; accepted for
compression surgery and reviewed the late follow-up echo-
publication May 15, 2009. cardiographic data to see whether the tricuspid valves
Address for reprints: Shunji Sano, MD, Professor and Chairman, Department of Car- continued to grow after biventricular repair.
diovascular Surgery, Okayama University Graduate School of Medicine, Dentistry
and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama, 700-8558, Japan
(E-mail: s_sano@cc.okayama-u.ac.jp).
J Thorac Cardiovasc Surg 2009;138:1160-6
PATIENTS AND METHODS
0022-5223/$36.00 Patient Population
Copyright ! 2009 by The American Association for Thoracic Surgery Between March 1993 and June 2006, we identified 40 infants with a
doi:10.1016/j.jtcvs.2009.05.015 diagnosis of PA–IVS who underwent RV decompression in our institution,

1160 The Journal of Thoracic and Cardiovascular Surgery c November 2009


Huang et al Congenital Heart Disease

and the incision in the pulmonary trunk. The ductus arteriosus was then li-
Abbreviations and Acronyms gated. The other 2 patients underwent percutaneous valvotomy and RV out-
flow tract patching as their initial treatments, respectively.
BSA ¼ body surface area
Secondary procedures. Diagnostic postoperative cardiac catheteri-
LVP ¼ left ventricular pressure zations were performed after the initial RV decompression surgery (n ¼ 33).
PA– IVS ¼ pulmonary atresia with intact The interval between operation and catheterization was 0.76 " 0.54 years.
ventricular septum At this stage, an RVP/LVP ratio greater than 0.5 was an indication for bal-
RV ¼ right ventricle (ventricular) loon pulmonary valvuloplasty (n ¼ 20). For patients with an RV end-dia-
stolic volume less than 50% of the predicted normal value estimated at
RVP ¼ right ventricular pressure
the last cardiac catheterization, we performed an ‘‘RV overhaul’’ procedure
t1 ¼ first time point (n ¼ 6).14,15 The RV overhaul procedure included repeat pulmonary valvot-
t2 ¼ second time point omy, transatrial and transpulmonary resection of the hypertrophied infun-
TV ¼ tricuspid valve dibular muscle, and adjustment of an interatrial communication. To
TVD ¼ tricuspid valve diameter increase blood flow through the tricuspid valve, we partially closed the atrial
septal defect, keeping right atrial pressure less than 15 mm Hg, and main-
Zt1 ¼ initial Z-value
tained a gradient across the atrial septal defect of less than 10 mm Hg.
Zt2 ¼ Z-value after treatment Definitive repair. We generally consider a tricuspid valve diameter
larger than 60% (Z ¼!6.3) of the normal mean as the indication for biven-
tricular repair. If the tricuspid valve is less than 50% of normal mean (Z
<!8.5), we consider single ventricle repair or one-and-a-half ventricle re-
and all were included in this study. Patients with critical pulmonary stenosis pair. The borderline cases (tricuspid valve size between 50% and 60% of

CHD
(pinhole patency of the pulmonary valve, suprasystemic pressures in the normal, Z ¼ !8.5 to !6.3) were judged on intraoperative findings. We
RV, and duct-dependent pulmonary circulation) and intact ventricular septa have the tendency to perform biventricular repair whenever possible. The
were also included (n ¼ 5) because, in all other respects, they were similar to surgical procedure included construction of an unobstructed RV outflow
patients with PA–IVS. Patients with no detectable infundibulum or RV- and closure of the intra-atrial communication and the systemic–pulmonary
dependent coronary circulation did not undergo RV decompression and shunt. We used various techniques including pulmonary valve repair, resec-
thus were not included in this study. tion of obstructing infundibular muscle, excision of hypertrophied RV mus-
cle to enlarge the cavity, and monocuspid transannular patching to achieve
Measurements a normal-sized outflow tract. During biventricular repair or one-and-a-half
We reviewed the echocardiographic and cardiac catheterization data. ventricle repair with a marginal tricuspid valve diameter, we used an adjust-
The tricuspid valve diameter was measured from echocardiograms at its able stitch to close the final interatrial communication defect for better judg-
maximum in diastole from the apical 4-chamber view. This tricuspid valve ment after weaning the patient from cardiopulmonary bypass. We left
diameter was then expressed as a percentage of the normal mean and the a small fenestration during closure of the interatrial septum in 14 patients,
Z-score obtained with the formula from the echocardiography-derived and 12 of them had spontaneous closure of the communication on follow-
normal value calculated using body surface area11: up echocardiograms.
After definitive repair, the patients were monitored echocardiographi-
lnðTVDÞ ¼ 1:08 þ 0:4945$lnðBSAÞ (1) cally. Among the 27 patients who underwent biventricular repair, cardiac
catheterization data were available for 17 and follow-up echocardiographic
where TVD ¼ tricuspid diameter (cm) and BSA ¼ body surface area (m2).
data were available for 24 patients.
Z-value ¼ ðln½observed TVD( ! ln½mean normal TVD(Þ=0:08121 (2)
Data Analysis and Statistical Methods
where TVD is as defined earlier. We compared the tricuspid valve Z-values at different time points to dis-
Direct measurements obtained during catheterization were used to esti- cern changes in tricuspid valve diameter. The first time point (t1) was de-
mate the RV/left ventricular systolic pressure (RVP/LVP) ratio. RV vol- fined as the Z-value obtained from the initial echocardiogram. The second
umes were calculated from biplane cineangiograms using the Simpson time point (t2) was defined as the Z-value just before definitive repair if
rule, as previously described.12 RV end-diastolic volume was corrected the patient underwent definitive repair. Otherwise, we chose the Z-value
for BSA and was expressed as a percentage of the predicted normal using from the most recent echocardiogram among patients in whom definitive re-
the formula derived by Nakazawa and colleagues.13 pair was not completed (ie, those with mixed systemic and pulmonary cir-
culation). The third time point (t3) was defined as the Z-value from the most
Predicted normal RV end-diastolic volumeðmLÞ ¼ 75:1
! "1:43 (3) recent echocardiogram among patients in whom biventricular repair was
3 BSA m2 completed. Disproportionate tricuspid valve growth was defined as the
change in tricuspid valve Z-values. Thus increase of Z-value indicated the
outgrowth of the tricuspid valve according to the BSA, and decrease of
Management of the Patients Z-value indicated the ‘‘shrinkage’’ of the tricuspid valve to the BSA.
All patients were treated with prostaglandin E1 infusion, and balloon To examine whether lower RVP influenced the growth of the tricuspid
atrial septostomy was performed during cardiac catheterization in most of valve, we examined the relationship of RVP/LVP after initial palliation to
the patients before surgery. differences in Z-scores of tricuspid valves. After biventricular repair, the he-
Initial procedures. Initial management was transarterial pulmonary modynamic data were reviewed and correlated with the size of the tricuspid
valvotomy and placement of a systemic–pulmonary shunt, without cardio- valve.
pulmonary bypass, as previously reported14 for the majority (95%, 38/40) Results are presented as means " SDs or percentages. The data were an-
of the patients. The pulmonary valve was penetrated and further opened alyzed with SPSS software (SPSS Inc, Chicago, Ill). The paired Student t
with a 3- or 4-mm Hegar dilator through the incision in the main pulmonary tests were used for comparing continuous variables for the same patient.
artery; a polytetrafluoroethylene tube (Gore-Tex tube; W. L. Gore & Asso- The Wilcoxon signed rank test was used for comparison of continuous vari-
ciates, Inc, Flagstaff, Ariz) was anastomosed to the left subclavian artery ables. Correlation was analyzed by linear correlation modeling, computing

The Journal of Thoracic and Cardiovascular Surgery c Volume 138, Number 5 1161
Congenital Heart Disease Huang et al

the Pearson correlation coefficient (r) and nonparametric regression analysis TABLE 1. The factors analyzed for the change of tricuspid valve
(categorical variables), as appropriate. Z-value (Zt2–Zt1)
Pearson Univariate
RESULTS Variables correlation P value
Demographic Data Initial evaluation (t1)
Among the 40 patients (21 boys and 19 girls), the mean Body surface area !0.018 .92
age at the first operation was 28.6 " 17.2 days; the average Z-value of TV !0.40 .02
body weight was 3.0 " 0.6 kg. RV volume (% of normal) !0.32 .13
There were 2 deaths after the stage I operations, and 1 pa- Tricuspid regurgitation !0.05 .79
tient was lost to follow-up. Two patients were waiting for Presence of sinusoid !0.307 (Spearman rho) .099
their next operations after initial valvotomy and shunt place- Age at first operation !0.11 .53
Second evaluation (t2)
ment. The remaining 35 patients received further treatment
Age at evaluation (t2) 0.27 .13
as follows: Four patients received bidirectional Glenn
RV volume (% of normal) !0.10 .58
shunts, and 1 of these infants died. Two patients were wait- RVP/LVP !0.54 .0013
ing for definitive repair after RV overhaul, and 29 patients TV, Tricuspid valve; RV, right ventricular; RVP, right ventricular pressure; LVP, left
underwent definitive repairs. ventricular pressure.
Mean patient age at definitive surgery was 2.4 " 0.9
years. The definitive repairs included biventricular repair
CHD

(n ¼ 27), Fontan circulation (n ¼ 1), and one-and-a-half ven- Comparing the tricuspid valve Z-value after treatment
tricle repair (n ¼ 1). One late death after biventricular repair (Zt2) with initial Z-value (Zt1) for individual patients showed
occurred 6 months after surgery; the cause of death was not that the change in Z-value (Zt2!Zt1) was larger thanþ2 in 11
clearly known (presumed arrhythmia). The overall median (32%) patients and smaller than!2 in 6 (18%) patients. The
follow-up period after the initial procedure was 6.8 years other 17 (50%) patients had Z-value changes within " 2. The
(1–13 years). increases in Z-values (Zt2 ! Zt1) were not related to patient
age at initial operation, presence of sinusoid communication,
Tricuspid Valve Growth or severity of tricuspid regurgitation (Table 1) The increases
There were 34 patients with Z-values at both t1 and t2 in Z-values (Zt2!Zt1) were significantly negatively associ-
available for analysis. The initial mean tricuspid valve Z- ated with RVP/LVP measured after initial palliation
value (Zt1) was !6.2 " 3.5, which corresponded to a Z- (Figure 2) (r ¼ !0.54; P ¼ .0013). The increases were also
value of !1.9 " 1.5 by autopsy-derived nomogram.16 After more marked among patients with initially small tricuspid
treatment (Zt2), the Z-value was!6.0 " 3.4 (Figure 1). The valve Z-values (r ¼!0.40; P ¼ .02) (Figure 3).
age at the t2 point was 2.2 " 1.1 years old. Overall, the Among the 27 patients in whom biventricular circulation
average of tricuspid valve Z-value did not change (P > was achieved, the mean initial Z-value (Zt1) was!5.8 " 3.6,
.05, paired Student t test). The RV volume increased and the mean Z-value was!4.9 " 2.63 (P<.05) when they
from 44% " 34% to 57% " 30% of the normal value received biventricular repair. The distributions of tricuspid
(P < 0.05). valve size with regard to 50% and 60% of normal value

FIGURE 2. The change of Z-value (Zt2–Zt1) showed negative correlation


FIGURE 1. The tricuspid valve Z-value at the initial evaluation (t1) and with the right ventricular pressure/left ventricular pressure (RVP/LVP)
after treatment (t2). measured after initial palliation (P ¼ .0013).

1162 The Journal of Thoracic and Cardiovascular Surgery c November 2009


Huang et al Congenital Heart Disease

FIGURE 3. The change of Z-value (Zt2 – Zt1) showed negative correla-


tion with the initial tricuspid valve Z-value (P ¼ .02).

FIGURE 4. Among the patients who received follow-up catherization after


(proposed criteria for biventricular repair) in these 27 pa- biventricular repair (n ¼ 17), the right atrial (RA) pressure is negatively

CHD
tients are depicted in Table 2. Among the 27 patients, 13 correlated with the tricuspid valve (TV) size after biventricular repair. The
(48%) had initial Z-values less than !6.3, and 7 of them Z-value was measured by echocardiography when the patient received fol-
low-up catheterization.
had increased Z-values to more than!6.3 at the stage of de-
finitive repair.
Seventeen patients underwent follow-up cardiac catheter- the RVP/LVP. This finding was never been well shown in
ization after definitive repairs (mean of 1.66 " 1.11 years af- previous studies, and some texts state that ‘‘there is no evi-
ter repair). Their right atrial pressures negatively correlated dence that disproportionate enlargement occurs as growth
with tricuspid valve Z-value (Pearson r ¼ !0.49; P ¼ .038) of the child proceeds, no matter what the surgical proce-
(Figure 4). All of these patients had right atrial pressures of dure.’’17 Even in the recent study by Bryant and associ-
less than 12 mm Hg, except for 1 patient with a right atrial ates,18 RV sinus myectomy was a proposed strategy for
pressure of 14 mm Hg. This particular patient had a tricuspid early biventricular repair. Although the RV size increased,
valve diameter equal to 48% of normal (Z-value ¼!9), but the Z-value of the tricuspid valve did not change apprecia-
she was considered a high-risk Fontan candidate because bly. However, the Z-value they compared (before and after
of left pulmonary vein occlusion after reimplantation for myectomy) started at the median age of 1.25 years, not the
anomalous return. She was still alive at 4 years of follow-up. neonatal periods, as in our cases.
Among the patients who underwent successful biventric- Because of the rarity of PA–IVS, single institution expe-
ular repairs, tricuspid valve Z-values further increased an rience is generally limited by the small number of cases; the
average of 1.14 " 1.09 by 4.9 " 3.2 years after definitive two largest studies are Congenital Heart Surgeons Society
surgery (P < .001, n ¼ 24). We could not find any specific studies.1,19 However, these studies did not provide data
parameter associated with the ‘‘enlargement’’ of the tricus- about late tricuspid valve diameter Z-values. Hanley and co-
pid valve after biventricular repair. workers1 summarized 53 patients from 4 studies.2,3,8,20 The
Z-values decreased without RV outflow tract procedures,
and in patients with RV outflow tract procedures, the overall
DISCUSSION
Z-value remained unchanged. It is of note that 15% of these
In this study, we found that the disproportionate growth of
patients experienced a Z-value change ofþ2 and 10% expe-
the tricuspid valve existed in some patients with PA–IVS
rienced a change of þ4, but this predictor of tricuspid valve
and related to the small initial tricuspid valve diameter and
growth was not known at the time of these studies.
TABLE 2. The distribution of tricuspid valve size among the 27 Similarly, we also found that the mean overall Z-value did
patients who received biventricular repair not change significantly, but the Z-value change was quite
Tricuspid valve size
variable among individual patient. We also found that lower
Z-value (% normal) RVP and smaller initial Z-values were associated with
greater increases in Z-values at their definitive repair.
Timing of Z <!8.5 !8.5 ) Z <!6.3 Z * !6.3
echocardiography (<50%) (50%–60%) (>60%)
Initial evaluation (t1) 6 7 14 RVP and Growth of Tricuspid Valve
At biventricular repair (t2) 2* 4 21 We looked for similar findings in other studies. In the
*The 2 patients have Z-values ¼ !10.2 and !9.7, respectively. report by Bull and associates21 in 1994, half of the

The Journal of Thoracic and Cardiovascular Surgery c Volume 138, Number 5 1163
Congenital Heart Disease Huang et al

TABLE 3. The changes of Z-value of the tricuspid valve among the


patients with pulmonary atresia–intact ventricular septum by the
right ventricular pressure after decompression procedure
Hypertensive RV Hypotensive RV
Increase in Z-value 6 6
Decrease in Z-value 8 3
No change in Z-value 1 1
Hypertensive RV, Suprasystemic right ventricular pressure; hypotensive RV, right ven-
tricular pressure lower than systemic pressure. (Adapted from Bull et al21 [Figure 5].)

decompressed RVs achieved tricuspid valve growth dispro-


portionate to somatic growth. Reviewing the presented
data,21 there were 15 patients with hypertensive RVs (supra-
systemic RVP) and 10 patients with hypotensive RVs after
RV decompression. The Z-value enlarged in only 40% of
the hypertensive RVs but in 60% of hypotensive RVs.
Thus, hypotensive RVs tended to have better tricuspid valve
growth, although this result did not reach statistical signifi-
CHD

cance (Table 3).


We combined the reports from Patel,3 Shaddy,10 and their
associates, in which the RVP and tricuspid valve diameter
were presented. We included patients with RV–pulmonary
artery continuity and recalculated the Z-values and the
changes in Z-values (n ¼ 26, one outlier was excluded).
We found that the change in Z-value was negatively corre-
lated with postoperative RVP (r ¼!0.552; P ¼ .003) and ini-
tial tricuspid valve Z-value (r ¼!0.579; P ¼ .002) (Figure 5).
These data support our finding that ‘‘disproportionate’’ FIGURE 5. The change in tricuspid valve Z-value in the patients reported
tricuspid valve growth occurred and that lower RVP and by Shaddy,10 Patel,3 and their associates. A, The higher right ventricular
smaller initial Z-value were predictors for better tricuspid (RV) pressure after initial decompression procedure is negatively correlated
valve growth. with tricuspid valve growth. B, The change in Z-value is also negatively cor-
The mechanisms of RV and tricuspid valve growth are not related with the initial tricuspid valve size (Initial Z-value). (Adapted from
Patel et al3 [Tables 1 and 2] and Shaddy et al10 [Table 2].)
clearly understood. Experimental pulmonary stenosis in fe-
tal lambs induced either greatly reduced or markedly in-
creased RV volume. Ventricular enlargement appears to be increments. This finding is also consistent with other find-
associated with a large ventricular volume load resulting ings in the literature. Percutaneous valvotomy was selec-
from tricuspid valve regurgitation. A small competent tricus- tively performed for patients with larger tricuspid valves.
pid valve is associated with reduced flow through the ventri- In the reports by both Humpl and associates5 (mean Z-value
cle owing to outflow obstruction and a small RV.22 This ¼ !1.33 " 1.58) and Ovaert and colleagues23 (Z-value ¼
experiment suggests that cardiac chamber development is !0.8; range: !1.3 to !0.4), the Z-values did not increase
influenced by blood flow, at least in the prenatal period. In and even decreased after percutaneous pulmonary valvot-
our study, lower RVP indicated a well-opened outflow tract omy. In the surgical series by Shaddy and coauthors,10 the
and, thus, increased flow through the RV; better tricuspid initial Z-value was !4 " 4, (73% " 21% of the normal
valve growth in patients with lower RVP is comparable mean), and these authors reported disproportional growth
with the flow-mediated growth theory. On the other hand, of the tricuspid valve in the majority of their patients. On
decreased RVP is beneficial for coronary perfusion to the the basis of these findings, we believe that the overall Z-
RV myocardium, regression of RV myocardial hypertrophy, value would not change if we included all patients, as in
and prevention of RV fibrosis. Furthermore, this mechanism the previous reports,1,14 and disproportionate growth was
possibly explains the better RV and tricuspid valve growth. possibly more frequently observed in patients with small
initial Z-values.
Initial Z-Value Relationship to Tricuspid Valve
Growth Z-Value of the Biventricular Circulation
Another interesting finding of our study is that smaller The definite tricuspid valve Z-value criterion for biven-
Z-values were associated with more pronounced Z-value tricular repair was not well established. Among the 27

1164 The Journal of Thoracic and Cardiovascular Surgery c November 2009


Huang et al Congenital Heart Disease

patients who received biventricular repair, 13 (48%) had an findings support the possibility of a neonate with a small tri-
initial Z-value less than!6.3, and the tricuspid valve size in- cuspid valve undergoing biventricular repair after RV de-
creased to more than!6.3 in 7 of them and between!6.3 and compression surgery.
!8.5 in the remaining 4 (Table 2) If we arbitrarily set the
Z-value greater than !6.3 as the criterion for biventricular We thank Dr. Fu-Chang Hu, National Center of Excellence for
repair, there will be 7 patients who became candidates for General Clinical Trial and Research, National Taiwan University
Hospital and College of Public Health, National Taiwan Univer-
biventricular repair despite the initial Z-value being less
sity, for his help in the statistical analysis.
than !6.3. We suggest not abandoning biventricular repair
in the neonatal period solely by the echocardiographic Z-
value of the tricuspid valve. In a recent report, Bryant and References
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CHD
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CHD

1166 The Journal of Thoracic and Cardiovascular Surgery c November 2009